1000 resultados para UDK:809
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Includes index.
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Mode of access: Internet.
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Por medio de la cual se amplían las posibilidades de retiro de cesantías para educación.
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Machado-Joseph disease (MJD) is the most common autosomal dominant spinocerebellar ataxia and presents great phenotypic variability. MJD presenting with spastic paraparesis was recently described in Japanese patients. We report the case of 41-year-old woman with the phenotype of complicated hereditary spastic paraplegia. Her father died at the age of 56 years due to an undiagnosed progressive neurological disease that presented parkinsonism. She had an expanded allele with 66 CAG repeats and a normal allele with 22 repeats in the gene of MJD. MJD should be considered in the differential diagnosis of autosomal dominant complicated HSP. A patient with the phenotype of complicated HSP and relatives with other clinical features of a neurodegenerative disease should raise the suspicion of MJD.
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The aim of this study was to evaluate whether yerba maté alcoholic extracts at very low concentrations (0.01 and 0.1%), prevent/retard lipid peroxidation in beef hamburgers without impairing sensory acceptability. For this TBARs and hexanal levels, fatty acid profile and cholesterol oxides were evaluated as oxidation parameters in beef hamburgers during 90 days' storage. The addition of 0.01% yerba maté ethanolic extracts proved inefficient in restraining the lipid peroxidation while the addition of 0.1% resulted in efficient antioxidant activity. Sensory evaluation of hamburger containing 0.1% yerba maté ethanolic extracts showed good acceptability. Yerba maté ethanolic extracts could entirely or partially replace the phenolic synthetic antioxidants in beef hamburgers only when used at above legally allowed concentrations for antioxidant additives (0.01%).
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Purpose: To facilitate future diagnosis of Knobloch syndrome (KS) and better understand its etiology, we sought to identify not yet described COL18A1 mutations in KS patients. In addition, we tested whether mutations in this gene lead to absence of the COL18A1 gene product and attempted to better characterize the functional effect of a previously reported missense mutation. Methods: Direct sequencing of COL18A1 exons was performed in KS patients from four unrelated pedigrees. We used immunofluorescent histochemistry in skin biopsies to evaluate the presence of type XVIII collagen in four KS patients carrying two already described mutations: c. 3277C>T, a nonsense mutation, and c. 3601G>A, a missense mutation. Furthermore, we determined the binding properties of the mutated endostatin domain p.A1381T (c.3601G>A) to extracellular matrix proteins using ELISA and surface plasmon resonance assays. Results: We identified four novel mutations in COL18A1, including a large deletion involving exon 41. Skin biopsies from KS patients revealed lack of type XVIII collagen in epithelial basement membranes and blood vessels. We also found a reduced affinity of p.A1381T endostatin to some extracellular matrix components. Conclusions: COL18A1 mutations involved in Knobloch syndrome have a distribution bias toward the coding exons of the C-terminal end. Large deletions must also be considered when point mutations are not identified in patients with characteristic KS phenotype. We report, for the first time, lack of type XVIII collagen in KS patients by immunofluorescent histochemistry in skin biopsy samples. As a final point, we suggest the employment of this technique as a preliminary and complementary test for diagnosis of KS in cases when mutation screening either does not detect mutations or reveals mutations of uncertain effect, such as the p.A1381T change.
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The aim of this work is to demonstrate the feasibility of laser induced breakdown spectrometry (LIBS) for the determination of macro and micronutrients in multielement tablets. The experimental setup was designed by using a laser Q-switch (Nd:YAG, 10 Hz, lambda = 1064 nm) and the emission signals were collected by lenses into an optical fiber coupled to an echelle spectrometer equipped with a high-resolution intensified charge coupled device (ICCD). Tablets were cryogenically ground and thereafter pelletized before LIBS analysis. Calibration curves were made by employing samples and mixtures of commercial multielement tablets with binders at different ratios. Best results were achieved by using the following experimental conditions: 29 J cm(-2) laser fluence, 165 mm lens to sample distance (f = 200 mm), 2.0 mu s delay time, 5.0 mu s integration time and 5 accumulated laser pulses. In general, the results obtained by the proposed LIBS procedure were in agreement with those obtained by ICP OES from the corresponding acid digests and coefficients variation of LIBS measurements varied from 2 to 16%. The metrological figures of merit indicate that LIBS fits for the intended purposes, and can be recommended for the analysis of multielement tablets and similar matrices aiming the determination of Ca, Cu, Fe, Mg, Mn, P and Zn.
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This work aims to evaluate the fermentability of cellulosic hydrolysates obtained by enzymatic saccharification of sugarcane bagasse pretreated by hydrothermal processing using Candida guilliermondii FTI 20037 yeast. The inoculum was obtained from yeast culture in a medium containing glucose as a carbon source supplemented with rice bran extract, CaCl(2)center dot 2H(2)O and (NH(4))(2)SO(4) in 50 mL Erlenmeyer flasks, containing 20 mL of medium, initial 5.5 pH under agitation of an orbital shaker (200 rpm) at 30A degrees C for 24 h. The cellulosic hydrolysates, prior to being used as a fermentation medium, were autoclaved for 15 min at 0.5 atm and supplemented with the same nutrients employed for the inoculum, except the glucose, using the same conditions for the inoculum, but with a period of 48 h. Preliminary results showed the highest consumption of glucose (97%) for all the hydrolysates, at 28 h of fermentation. The highest concentration of ethanol (20.5 g/L) was found in the procedure of sugarcane bagasse pretreated by hydrothermal processing (195A degrees C/10 min in 20 L reactor) and delignificated with NaOH 1.0% (w/v), 100A degrees C, 1 h in 500 mL stainless steel ampoules immersed in an oil bath.
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This paper addresses the development of several alternative novel hybrid/multi-field variational formulations of the geometrically exact three-dimensional elastostatic beam boundary-value problem. In the framework of the complementary energy-based formulations, a Legendre transformation is used to introduce the complementary energy density in the variational statements as a function of stresses only. The corresponding variational principles are shown to feature stationarity within the framework of the boundary-value problem. Both weak and linearized weak forms of the principles are presented. The main features of the principles are highlighted, giving special emphasis to their relationships from both theoretical and computational standpoints. (C) 2010 Elsevier Ltd. All rights reserved.
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Prevalence and comorbidity of behavioral problems of children aged three to six: Results of the Braunschweiger Kindergartenstudie Objectives: To analyze the frequency of behavioral and emotional problems and comorbidity of kindergarten children in Braunschweig as rated by their parents. Method: The analysis is part of the Braunschweiger Kindergartenstudie. In a sample of N = 809 children aged three to six the parents rated their children using a modified version of the Child Behavior Checklist/CBCL 4-18. Results: The prevalence rates range from 0.5% to 5.0%. The most frequent behavioral problems in kindergarten children were aggressive behavior and attention problems, followed by social problems. The study also provides bidirectional comorbidity rates. Conclusion: Finally the prevalence rates and the implications of the findings for prevention of behavioral problems in children are discussed.
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This paper outlines approaches to developing the International Society of Physical and Rehabilitation Medicine (ISPRM) and addresses many current challenges Most importantly, these approaches provide the basis for ISPRM to develop its leadership role within the field of Physical and Rehabilitation Medicine (PRM) and in relation to the World Health Organization (WHO) and the United Nations (UN) system at large. They also address a number of specific critiques of the current situation. A positioning of ISPRM within the world architecture of the UN and WHO systems, as well as the consideration and fostering of respective emerging regional PRM societies, is central to establishing networking connections at different levels of the world society. Yearly congresses, possibly in co-operation with a regional society, based on a defined regional rotation, are suggested. Thus, frustration with the current bidding system for a biennial congress and an intermediate meeting could be overcome. Yearly congresses are also an important step towards increasing the organization`s funding base, and hence the possibility to expand the functions of ISPRM`s central office. ISPRM`s envisioned leadership role in the context of an international web of PRM journals complementing the formally defined official journal of ISPRM, regional societies and so forth, is an inclusive rather than exclusive approach that contributes to the development of PRM journals worldwide. An important prerequisite for the further development of ISPRM is the expansion and bureaucratization of its Central Office, adding professionalism and systematic allocation of resources to the strengths of the voluntary engagement of individual PRM doctors.
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Schistosoma mansoni is responsible for the neglected tropical disease schistosomiasis that affects 210 million people in 76 countries. Here we present analysis of the 363 megabase nuclear genome of the blood fluke. It encodes at least 11,809 genes, with an unusual intron size distribution, and new families of micro-exon genes that undergo frequent alternative splicing. As the first sequenced flatworm, and a representative of the Lophotrochozoa, it offers insights into early events in the evolution of the animals, including the development of a body pattern with bilateral symmetry, and the development of tissues into organs. Our analysis has been informed by the need to find new drug targets. The deficits in lipid metabolism that make schistosomes dependent on the host are revealed, and the identification of membrane receptors, ion channels and more than 300 proteases provide new insights into the biology of the life cycle and new targets. Bioinformatics approaches have identified metabolic chokepoints, and a chemogenomic screen has pinpointed schistosome proteins for which existing drugs may be active. The information generated provides an invaluable resource for the research community to develop much needed new control tools for the treatment and eradication of this important and neglected disease.
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A barracuda implicated in ciguatera fish poisoning in Guadeloupe was estimated to have an overall flesh toxicity of 15 MUg/g using mouse bioassay. A lipid soluble extract was separated into two toxic fractions, FrA and FrB, on a LH20 Sephadex column eluted with dichloromethane/methanol (1:1). When intraperitoneal injected into mice, FrA provoked symptoms characteristic of slow-acting ciguatoxins, whereas FrB produced symptoms indicative of fast-acting toxins (FAT). High performance liquid chromatography/mass spectrometry/radio-ligand binding (HPLC/MS/RLB) analysis confirmed the two fractions were distinct, because only a weak overlap of some compounds was observed. HPLC/MS/RLB analysis revealed C-CTX-1 as the potent toxin present in FrA, and two coeluting active compounds at m/z 809.43 and 857.42 in FrB, all displaying the characteristic pattern of ion formation for hydroxy-polyethers. Other C-CTX congeners and putative hydroxy-polyether-like compounds were detected in both fractions, however, the RLB found them inactive. C-CTX-1 accounted for >90% of total toxicity in this barracuda and was confirmed to be a competitive inhibitor of brevetoxin binding to voltage-sensitive sodium channels (VSSCs) with a potency two-times lower than P-CTX-1. However, FAT active on VSSCs and
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O texto incorpora uma diversidade de ferramentas de planejamento estrat??gico. Sua principal caracter??stica ?? a de ver o planejamento estrat??gico como um processo e n??o apenas como um projeto que se finda com a antevis??o de cen??rios prospectivos e suas poss??veis solu????es estrat??gicas.